Iron-sulfur cluster assembly 1 homolog, mitochondrial (ISCA1)

Iron-sulfur cluster assembly 1 homolog, mitochondrial (ISCA1) is an evolutionarily conserved mitochondrial carrier protein required for forming and inserting [4Fe-4S] iron-sulfur clusters into mitochondrial proteins. It functions as a late component in the ISC biogenesis pathway, working alongside ISCA2 and IBA57, and is essential for the maturation of key mitochondrial enzymes, such as aconitase and components of respiratory chain complexes. Genetic knockout of ISCA1 in animal models causes embryonic death, and human mutations result in devastating mitochondrial syndromes marked by neurodegeneration, myopathy, and metabolic failure. To date, no drugs target ISCA1 directly, but its dysfunction serves as a biomarker for several inherited mitochondrial diseases. Its critical roles in mitochondrial biogenesis and energy metabolism make it a research focus for understanding and treating metabolic disorders[1][2][3][4][5].

Other names

ISCA1Iron-sulfur cluster assembly 1 homolog, mitochondrialHESB-like domain-containing protein 2 (HBLD2)Iron-sulfur assembly protein IscAGK004hIscAISA1hIscA1MMDS5MGC4276

Not applicable; no drugs directly targeting ISCA1 are currently described. Investigational approaches may include gene therapy or agents affecting mitochondrial Fe/S cluster biogenesis, but these remain experimental[2][3][5].